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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Metastatic behavior of neuroblastic tumors was analyzed to determine whether the secondary distribution of tumor was random, or whether the pattern of metastases was predictable and related to tumor type. The authors reviewed the 64 patients subjected to complete autopsy who had metastases from a neuroblastoma, retinoblastoma, medulloblastoma, or pinealoblastoma. The organ and tissue distribution of metastases was recorded in relation to location of primary tumor, type of therapy, survival, and presence of central nervous system metastases. Data were analyzed using chi-square contingency tables, correlation coefficients, and cluster analysis. The results indicate that: (1) the development of central nervous system metastases from neuroblastoma correlates with the pattern of extracentral nervous system metastases; (2) the pattern of tumor metastases is altered by chemotherapy and/or radiation; and (3) regardless of the site of origin, neuroblastic tumors behave in a uniform manner with a nonrandom metastatic distribution in particular tissues within the central nervous system. Cluster analysis demonstrated patterns of neuroblastoma tumor metastases which were consistent with both Hutchison and Pepper syndromes. The findings suggest that a random distribution, as might be secondary to blood flow, does not account for metastatic patterns, but rather that there are preferential patterns of growth, possibly reflecting trophic tendencies in neoplasms.
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PMID:Nonrandom distribution of metastases in neuroblastic tumors. 687 32

From our study of eight pineoblastomas and five pineocytomas and a review of the literature, we have described two clinicopathologic syndromes that characterize these neoplasms. Pineoblastomas highly resemble the medulloblastoma-neuroblastoma group of tumors and occur mostly in young people. The tempo of progression of the disease is fast, the length of illness is short. These are infiltrating neoplasms that commonly spread via the cerebrospinal fluid. They are radiosensitive. Histologically they are also similar to the medulloblastoma-neuroblastoma group and are characterized by the scarcity of cytoplasmic processes and by the Homer Wright rosette. They contain giant cells. Pineocytomas are tumors of adults. The tempo of progression of the disease is slow, and the length of illness is long. They expand by compressing the surrounding tissues. Histologically they are characterized by the abundance of cytoplasmic processes and by the pineocytomatous rosette. They contain giant cells. Areas composed of neoplastic gangliocytes and astrocytes in various combinations are common variants in some of these neoplasms.
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PMID:The separation of pineocytoma from pineoblastoma. 698 79

Classic medulloblastoma is a relatively common and distinct clinicopathologic entity consisting of primitive multipotential cells with differentiating capacity to neuroblastic and/or glial cell lines. Desmoplastic medulloblastoma has some features in common with cerebellar neuroblastoma, in which ultrastructural evidence of significant neuroblastic differentiation is extremely rare. We studied three cerebellar tumors with evidence of neuronal differentiation as compared to four classical desmoplastic medulloblastomas. Two of three tumors contained the regions of different degrees resembling desmoplastic medulloblastoma and one consisted of neuroblastic cells exclusively. This spectrum of differentiation suggests a relationship between cerebellar neuroblastoma and medulloblastoma, especially of the desmoplastic type. The nature of cerebellar neuroblastoma and its nosology are briefly discussed.
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PMID:Morphological investigations on cerebellar "neuroblastoma" group. 706 56

Analysis of the CT findings in 35 cases of tumoral hemorrhage (taken from 973 intracranial tumors) revealed three distinct patterns of bleeding: (a) hematoma, (b) central hemorrhage, and (c) hemorrhagic infarction. The location, multiplicity of lesions, and contrast enhancement are important in the diagnosis, and the clinical history and arteriography may also be helpful. The largest single group in this series consisted of 12 metastatic lesions: the others included glioblastoma (7), chromophobe adenoma (4), Grade I astrocytoma (3), medulloblastoma (3), central neuroblastoma (2), histiocytic lymphoma (2), and ependymoma (1). The relatively low mortality rate (21/35) despite marked neurological deterioration is attributed to prompt CT demonstration of hemorrhage followed by aggressive therapy (surgical evacuation, total resection, radiotherapy, and/or steroids or mannitol).
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PMID:Computed tomography of acute intratumoral hemorrhage. 736 26

The expression of low-affinity nerve growth factor receptor (NGF-R) by primitive neuroectodermal tumors (PNETs) was analyzed in vivo and in vitro to investigate the relationship between NGF-R expression and cellular differentiation. NGF-R was expressed in one medulloblastoma cell line and two neuroblastoma cell lines. When these cells were induced to differentiate by treatment with dibutyryl cyclic adenosine monophosphate, NGF-R was overexpressed and there was increased expression of neurofilament proteins. Immunohistochemistry investigation of tumor tissues demonstrated that NGF-R was expressed by a subset of PNETs with a neuronal phenotype marked by neurofilament protein expression, but not by gliomas and PNETs without a neuronal phenotype. Growth inhibition assay demonstrated that NGF inhibited the growth of cells expressing NGF-R. These results indicate that NGF-R expression is a useful marker of neuronal differentiation by PNETs.
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PMID:Expression of nerve growth factor receptor by human primitive neuroectodermal tumors. 752 37

This study records the disease profile and outcome of all 492 children with confirmed cancer below the age of 15 who were admitted to Tygerberg Hospital, South Africa, from 1983 to 1993. The black (48.3%), so-called coloured (30.3%) and caucasian (21.3%) children did not represent a confined geographical area. Leukaemia (22.8%), brain tumours (20.5%), lymphomas (15.2%), nephroblastomas (10%), neuroblastomas (8.5%) and retinoblastomas (5.7%) were the most common tumours. All children were treated with standard protocols and included in the Kaplan-Meier survival analyses. 14 patients were lost to follow-up. Projected survival in (acute lymphoblastic leukaemia) ALL was 63% in white children, but only 17% in black children. Survival was 65% in stage 1 and 2 Wilms' tumour, and exceeded 50% in medulloblastoma and astrocytoma. So-called African Burkitt's lymphoma occurred in all population groups. Overall, 5-year survival in Hodgkin's disease was 70%. Black and coloured children with neuroblastoma presented mainly with stage 3 and 4 disease. All 26 black and coloured children with retinoblastoma had a negative family history and advanced disease which needed enucleation.
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PMID:The Tygerberg Hospital Children's Tumour Registry 1983-1993. 757 74

The authors investigated the effects of a nontoxic differentiation inducer, phenylacetate (PA), on neuroectodermal tumor-derived cell lines. Treatment of medulloblastoma (Daoy and D283 MED) and glioma (U-251MG, C6, and RG2) cell lines resulted in a dose-dependent decline in DNA synthesis and cell proliferation, associated with accumulation in the G0/G1 phase of the cell cycle. Phenylacetate decreased transforming growth factor (TGF)-beta 2 production by medulloblastoma Daoy cells. Neutralizing antibodies against either TGF beta 2 or TGF beta 1 failed to block the growth arrest observed. This suggests that, unlike other differentiation agents, such as retinoic acid, the effect of PA on medulloblastoma proliferation is not mediated by a TGF beta pathway. In addition to cytostasis, PA induced marked morphological changes in U-251MG and C6 glioma cells associated with increased abundance of glial fibrillary acidic protein-positive processes. Although the morphology of PA-treated medulloblastoma cells was not significantly altered, the D283 MED cells exhibited increased expression of neurofilament proteins and Hu antigen, indicative of differentiation along a neuronal pathway. The effects of PA on the medulloblastoma cell lines were compared to its effects on the human neuroblastoma cell line BE(2)C, which is capable of a bidirectional differentiation toward a neuronal or a glial/schwann cell pathway. In BE(2)C cells, PA induced differentiation toward a schwann/glial cell-like phenotype, suggesting that the choice of differentiation pathway is cell type and agent specific. These in vitro antiproliferative and differentiation inducing effects of PA suggest that this agent warrants further evaluation as a potential therapeutic modality for the treatment of medulloblastoma and malignant glioma in humans.
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PMID:Inhibition of proliferation and induction of differentiation in medulloblastoma- and astrocytoma-derived cell lines with phenylacetate. 767 18

Five brain tumors in infancy were studied histopathologically. Though four of them could be included in primitive neuroectodermal tumor, histopathological findings were diverse. The diagnoses were medulloblastoma, cerebellar neuroblastoma, pineoblastoma, ependymoblastoma and ependymoma. While conventional stain for microscopy exhibited their specific findings, immunohistochemical and electron microscopical studies revealed morphological findings related functional structures of the tumor cells. These methodology is mandatory in order to elucidate cytogenesis and differentiation of infantile brain tumors.
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PMID:Pathology of infantile brain tumor. 769 30

The 14 tumors reported in Rubinstein-Taybi syndrome since 1989, when added to the 22 previously reported, are beginning to show a pattern of neural and developmental tumors, especially of the head, which is malformed in the syndrome. Among the neoplasms were 12 of the nervous system: 2 each of oligodendroglioma, medulloblastoma, neuroblastoma, and benign meningioma, a pheochromocytoma, and 3 other benign tumors; 2 of nasopharyngeal rhabdomyosarcoma; and 1 each of leiomyosarcoma, seminoma, and embryonal carcinoma. Among the other benign tumors were an odontoma, a choristoma, a dermoid cyst, and 2 pilomatrixomas.
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PMID:Tumors in Rubinstein-Taybi syndrome. 955 2

Multidrug resistance represents one of the most important factors that may lead to a therapeutic failure in some patients affected by malignancies. One of the best known mechanisms is linked to the genic amplification or the overproduction of a membrane glycoprotein, GP170, that is the product of the gene MDR1. The existence of drugs (calcium blockers, cyclosporine, tamoxifen, reserpine, quinidine) able to bind themselves to gp170 and to paralyze its activity in vitro is well known. We studied 20 pediatric patients (median age 9 years) affected by acute lymphoblastic leukemia (ALL), osteosarcoma, neuroblastoma and medulloblastoma, in advanced stage of disease. We employed in all cases the association of cytostatics with verapamil (50-70 mg/m2 i.v.) and cyclosporine (5-8 mg/kg i.v.) with different infusion schedules. In leukemias we administered vincristine (1.5 mg/m2), and daunomycin (40 mg/m2), in solid tumors VP16 (150 mg/m2) and adriamycin (60 mg/m2). Seventy-two therapeutic courses were performed: 39 in ALL, 16 in osteosarcoma, 16 in neuroblastoma and 1 in medulloblastoma. On the whole 5 complete remissions were achieved in ALL patients and 1 in an osteosarcoma patient. We did not observe a significant myelosuppression during treatment, therefore few infectious complications occurred; furthermore electrocardiographic changes have been mild and promptly resolved after temporary discontinuation of verapamil infusion. Our data suggest a synergy of verapamil and cyclosporine in the inhibition of multidrug resistance induced by gp170, without the occurrence of heavy toxicity. The results obtained in ALL patients are encouraging., especially in view of a possible subsequent bone marrow transplantation, while in solid tumors they are not as satisfying.
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PMID:[Use of cyclosporin and verapamil in association with chemotherapy in the treatment of pediatric patients with advanced-stage neoplasms. A pilot study]. 780 68


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